This case report describes two patients with a very rare condition who presented with pathologic symptoms. Anomalous origin of the left coronary artery from the noncoronary cusp has been described as a "benign" lesion by some authors in the past, although rare cases of morbidity/mortality are described in the literature. Both reported patients underwent surgical repair for the lesion and at this writing are asymptomatic at follow-up evaluation. These two patients presenting with pathologic symptoms and undergoing surgery afford novel descriptions. The authors believe these descriptions add to our knowledge of this rare disorder.